154
Table 10.1 Classification of muscular involvement in primary Sjögren’s syndrome
G.J. Pons-Estel et al.
1. Myalgias (33%) [25]
2. Fibromyalgia (47–55%) [26, 27]
3. Subclinical myositis (72%) [28]
4. Myositis similar to PM (2.5–10%) [29] 5. Inclusion body myositis-like (22%) [28]
vacuolar changes is possibly related to the increased expression of membrane attack complex suggesting pathogenetic similarities to dermatomyositis. A classification of muscular involvement in primary Sjögren’s syndrome is shown in Table 10.1.
Considering all these data together, muscle biopsy is not justified in patients with primary Sjögren’s syndrome regardless of the presence or absence of muscle pain; however, the presence of FM does not rule out PM. Thus, biopsy should be considered in patients with primary Sjögren’s syndrome who exhibit significant muscle weakness whether FM manifestations are present or not.
References
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2. Ioannidis J, Vassiliou V, Moutsopoulos H. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum. 2002;46:741–7.
3. Theander E, Manthorpe R, Jacobsson L. Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum. 2004;50:1262–9.
4. Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, et al. Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford). 2006;45:187–91.
5. Ramos-Casals M, Solans R, Rosas J, et al. Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008;87:210–9.
6. Haga HJ, Peen E. A study of the arthritis pattern in primary Sjögren’s syndrome. Clin Exp Rheumatol. 2007;25:88–91.
7. Kruize AA, Hené RJ, Van Der Heide A, et al. Long-term followup of patients with Sjögren’s syndrome. Arthritis Rheum. 1996;39:297–303.
8.Haga HJ, Jonsson R. The influence of age on disease manifestations and serological characteristics in primary Sjögren’s syndrome. Scand J Rheumatol. 1999;28:227–32.
9. Ramos-Casals M, Cervera R, Font J, et al. Young onset of primary Sjögren’s syndrome: clinical and immunological characteristics. Lupus. 1998;7:202–6.
10. García-Carrasco M, Cervera R, Rosas J, et al. Primary Sjögren’s syndrome in the elderly: clinical and immunological characteristics. Lupus. 1999;8:20–3.
11. Barrs DM, McDonald TJ, Duffy J. Sjögren’s syndrome involving the larynx: report of a case. J Laryngol Otol. 1979;93:933–6.
12. Sève P, Poupart M, Bui-Xuan C, et al. Cricoarytenoid arthritis in Sjögren’s syndrome. Rheumatol Int. 2005;25:301–2.
13. García-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). 2002;81:270–80.
14. Kamali S, Polat NG, Kasapoglu E, et al. Anti-CCP and antikeratin antibodies in rheumatoid arthritis, primary Sjögren’s syndrome, and Wegener’s granulomatosis. Clin Rheumatol. 2005;24:673–6.
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15.Atzeni F, Sarzi-Puttini P, Lama N, et al. Anti-cyclic citrullinated peptide antibodies in primary Sjögren syndrome may be associated with non-erosive synovitis. Arthritis Res Ther. 2008;10:1–6.
16. Papiris SA, Tsonis IA, Moutsopoulos HM. Sjögren’s syndrome. Semin Respir Crit Care Med. 2007;28:459–71.
17. Rehman HU. Sjögren’s syndrome. Yonsei Med J. 2003;44:947–54.
18. Castro-Poltronieri A, Alarcón-Segovia D. Articular manifestations of primary Sjögren’s syndrome. J Rheumatol. 1983;10:485–8.
19. Rantapaa-Dahlqvist SR, de Jong BA, Berglin E, et al. Antibodies against cyclic citrullinated peptides and IgA rheumatoid factor predict the development of rheumatoid arthritis. Arthritis Rheum. 2003;48:2741–9.
20. Gottenberg JE, Mignot S, Nicaise-Rolland P, et al. Prevalence of anti-cyclic citrullinated peptide and anti-keratin antibodies in patients with primary Sjögren’s syndrome. Ann Rheum Dis. 2005;64:114–7.
21. Goëb V, Salle V, Duhaut P, et al. Clinical significance of autoantibodies recognizing Sjögren’s syndrome A (SSA), SSB, calpastatin and alpha-fodrin in primary Sjögren’s syndrome. Clin Exp Immunol. 2007;148:281–7.
22. Cervera R, Khamashta MA, Font J, et al. Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European working party on systemic lupus erythematosus. Medicine (Baltimore). 1993;72:113–24.
23. Spilberg I, Siltzbach LE, McEwen C. The arthritis of sarcoidosis. Arthritis Rheum. 1969;12:126–37.
24. Petri MA. In: Koopman WJ, editor. Arthritis and allied conditions – a textbook of rheumatology. 14th ed. Philadelphia: Lippincott Williams & Wilkin; 2001. p. 1455–79.
25. Martinez-Lavin M, Vaughan JH, Tan EM. Autoantibodies and the spectrum of Sjögren’s syndrome. Ann Intern Med. 1979;91:185–90.
26.Vitali C, Tavoni A, Neri R, et al. Fibromyalgia features in patients with primary Sjögren’s syndrome. Evidence of a relationship with psychological depression. Scand J Rheumatol. 1989;18:21–7.
27. Tishler M, Barak Y, Paran D, et al. Sleep disturbances, fibromyalgia and primary Sjögren’s syndrome. Clin Exp Rheumatol. 1997;15:71–4.
28. Lindvall B, Bengtsson A, Ernerudh J, et al. Subclinical myositis is common in primary Sjögren’s syndrome and is not related to muscle pain. J Rheumatol. 2002;29:717–25.
29. Alexander EL. Neuromuscular complications of primary Sjögren’s syndrome. In: Talal N, Moutsopoulos HM, Kassan SS, editors. Sjögren’s syndrome. Clinical and immunological aspects. Berlin: Springer; 1987. p. 61–82.
30. Chad D, Good P, Adelman L, et al. Inclusion body myositis associated with Sjögren’s syndrome. Arch Neurol. 1982;39:186–8.
31. Gutmann L, Govindan S, Riggs JE, et al. Inclusion body myositis and Sjögren’s syndrome. Arch Neurol. 1985;42:1021–2.
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34. Kraus A, Cifuentes M, Villa AR, et al. Myositis in primary Sjögren’s syndrome. Report of 3 cases. J Rheumatol. 1994;21:649–53.
35. Molina R, Provost TT, Arnett FC, et al. Primary Sjögren’s syndrome in men. Clinical, serologic, and immunogenetic features. Am J Med. 1986;80:23–31.
36. Ringel SP, Forstot JZ, Tan EM, et al. Sjögren’s syndrome and polymyositis or dermatomyositis. Arch Neurol. 1982;39:157–63.
37. Layzer RB. Inflammatory and immune disorders. In: Layzer RB, editor. Neuromuscular manifestations of systemic disease. Philadelphia: Davis; 1985. p. 227–8.
38. Leroy JP, Drosos AA, Yiannopoulos DI, et al. Intravenous pulse cyclophosphamide therapy in myositis and Sjögren’s syndrome. Arthritis Rheum. 1990;33:1579–81.
Chapter 11
Non-Vasculitic Cutaneous Involvement
Hobart W. Walling and Richard D. Sontheimer
Contents
11.1 |
Introduction................................................................................................................. |
157 |
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11.2 |
Epidemiology ............................................................................................................... |
160 |
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11.3 |
Skin Changes Encountered in Primary SjS.............................................................. |
161 |
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11.3.1 |
Pruritus ............................................................................................................ |
162 |
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11.3.2 Annular Erythema of SjS ................................................................................ |
162 |
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11.3.3 |
Eyelid Dermatitis ............................................................................................ |
164 |
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11.3.4 |
Panniculitis...................................................................................................... |
165 |
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11.3.5 Primary Nodular Cutaneous Amyloidosis ...................................................... |
165 |
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11.3.6 |
B Cell Lymphoma ........................................................................................... |
166 |
11.4 |
Skin Changes Encountered in Secondary SjS .......................................................... |
168 |
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11.4.1 Skin Changes Associated with Lupus Erythematosus .................................... |
168 |
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11.4.2Skin Changes Associated with Rheumatoid Arthritis, Systemic
Sclerosis, and the Idiopathic Inflammatory Myopathies................................. |
169 |
References............................................................................................................................... |
169 |
11.1Introduction
As with other clinically and genetically heterogeneous, multisystem autoimmune disorders, cutaneous abnormalities are frequently encountered in Sjogren’s syndrome (SjS). Thus, it is important to have a rational framework for identifying and treating skin changes that can be encountered in SjS patients and to understand the relationships of key skin changes to the SjS systemic disease process.
H.W. Walling
Private Practice of Dermatology, Coralville, IA, USA
R.D. Sontheimer (*)
Department of Dermatology, University of Utah School of Medicine, Salt Lake City, UT, USA
M. Ramos-Casals et al. (eds.), Sjögren’s Syndrome, |
157 |
DOI 10.1007/978-0-85729-947-5_11, © Springer-Verlag London Limited 2012 |
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158 |
H.W. Walling and R.D. Sontheimer |
Table 11.1 A Classification framework for the various types of skin change that can be encountered in SjS patients
Skin changes that are thought to relate directly from the underlying systemic autoimmune abnormalities of SjSa
Dryness (syn. xerosis, astetosis)
Skin changes associated with mucosal dryness Eyelid dermatitis
Angular cheilitis Pruritus
Annular erythema of Sjögren’s syndrome Erythema multiforme-like lesions Doughnut-like lesions
Sweet’s syndrome-like lesions Erythema perstans-like lesions
Panniculitis
Erythema nodosum
Necrotizing lymphocytic panniculitis Granulomatous panniculitis
Primary nodular cutaneous amyloidosis Cutaneous B cell lymphoma
Skin changes seen in other systemic autoimmune disorders that can overlap with SjSa
Rheumatoid arthritis Rheumatoid nodules
Rheumatoid papules (syn. rheumatoid neutrophilic dermatosis, interstitial granulomatous dermatitis, palisading neutrophilic granulomatous dermatitis)
Lupus erythematosus
Photosensitive malar rash of acute cutaneous LE Subacute cutaneous LE
Classical discoid LE
Chilblains LE (syn. perniotic LE, “perniosis”) Neonatal LE skin lesions in infants of mothers with SjS
Systemic sclerosis
Hand and finger changes Raynaud’s phenomenon Sclerodactly
Periungual nalilfold changes (capillary dropout, ectasia, and arborization; cuticular hemorrhages, ragged cuticles [Samitz sign])
Proximal extremity, trunkal and facial scleroderma Cutaneous calcinosis
Salt and pepper pattern of cutaneous dyspigmentation Dermatomyositis
Hallmark cutaneous manifestations of DM Periorbital heliotrope erythema Göttron’s papules
Göttron’s sign of DM (violaceous erythema of the extensor surfaces of the upper and lower extremities, especially the elbows and knees)
Violaceous erythema of the scalp
Violaceous erythema over the nape of the neck and posterior upper shoulders (shawl sign) Violaceous erythema over the lateral trochanteric of the hips (holster sign)
Cutaneous calcinosis
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Table 11.1 (continued)
Skin changes associated with the 8.1 ancestral haplotype that can be fortituously encountered in SjS patientsb
Alopecia areata Vitiligo
Skin changes reported anecdotally in SjS patients for which a significant association has yet to be confirmedb
Amicrobial pustulosis Cutaneous T cell lymphoma
Disseminated superficial actinic porokeratosis Erythema elevatum diutinum
Erythema nodosum
Erythematous swelling of the lip mimicking cheilitis granulomatosa Lichen planus
Livedoid vasculopathy Multiple dermatofibromas
Painful indurated erythema (Kikuchi-Fujimoto disease) Postmenopausal frontal fibrosing alopecia
Primary anetoderma Psoriasis
Pyoderma gangrenosum Subcorneal pustular dermatosis Sweet’s syndrome
Ulcerative lichen planus
Skin changes that can result from the treatment of SjSb
Corticosteroid-induced acne Antimalarial-induced skin changes
Hyperpigmentation Hypersensitivity reactions
Acute generalized exanthematous pustulosis Eczematous reactions
Exanthematous reactions Erythema multiforme reactions
Lichenoid drug eruptions (including subacute cutaneous LE-like reactions)
aListed in relative descending order of prevalence bListed in alphabetical order
The focus of this chapter will be on the cutaneous manifestations of primary SjS. However, a number of other types of skin change can be encountered in SjS patients (see Table 11.1). To better recognize and appreciate the skin disorders that are thought to relate directly to the systemic autoimmune process associated with SjS, one must be aware of the full spectrum of skin changes that can be encountered in SjS patients. We therefore have provided a framework for considering the entire spectrum of skin change that can be encountered in SjS patients (Table 11.1).
One such set of cutaneous changes that can be encountered in SjS are those that the overlapping autoimmune entities seen in secondary SjS patients can “bring along.” Examples include subacute cutaneous lupus erythematosus (SCLE) and